title: Brainstem manifestations in neuromyelitis optica: a multicenter study of 258 patients.
abstract: Neuromyelitis optica (NMO) is a severe autoimmune disease of the central nervous system characterized by spinal cord and optic nerve involvement. Brainstem manifestations have recently been described.
  
  
  
  To evaluate the time of occurrence, the frequency and the characteristics of brainstem symptoms in a cohort of patients with NMO according to the ethnic background and the serologic status for anti-aquaporin-4 antibodies (AQP4-abs).
  
  
  
  We performed a multicenter study of 258 patients with NMO according to the 2006 Wingerchuk criteria and we evaluated prospectively the frequency, the date of onset and the duration of various brainstem signs in this population.
  
  
  
  Brainstem signs were observed in 81 patients (31.4%). The most frequently observed signs were vomiting (33.1%), hiccups (22.3%), oculomotor dysfunction (19.8%), pruritus (12.4%), followed by hearing loss (2.5%), facial palsy (2.5%), vertigo or vestibular ataxia (1.7%), trigeminal neuralgia (2.5%) and other cranial nerve signs (3.3%). They were inaugural in 44 patients (54.3%). The prevalence was higher in the non-Caucasian population (36.6%) than in the Caucasian population (26%) (p<0.05) and was higher in AQP4-ab-seropositive patients (32.7%) than in seronegative patients (26%) (not significant).
  
  
  
  This study confirms the high frequency of brainstem symptoms in NMO with a majority of vomiting and hiccups. The prevalence of these manifestations was higher in the non Caucasian population.
authors.affiliation: Department of Neurology, University Hospital of Strasbourg, France laurentdaniel.kremer@chru-strasbourg.fr.
authors.forename: L
authors.initials: L
authors.lastname: Kremer
authors.affiliation: Department of Neurology, John Hopkins University, USA.
authors.forename: M
authors.initials: M
authors.lastname: Mealy
authors.affiliation: The Walton Centre for Neurology and Neurosurgery, UK.
authors.forename: A
authors.initials: A
authors.lastname: Jacob
authors.affiliation: Department of Neurology, Tohoku University, Japan.
authors.forename: I
authors.initials: I
authors.lastname: Nakashima
authors.affiliation: Department of Neurology, Centre Hospitalier La Meynard, France.
authors.forename: P
authors.initials: P
authors.lastname: Cabre
authors.affiliation: Division of Neurology, The Hospital for Sick Children, Toronto, Canada.
authors.forename: S
authors.initials: S
authors.lastname: Bigi
authors.affiliation: Department of Neurology and NeuroCure Clinical Research Center, Charité University Medicine, Germany.
authors.forename: F
authors.initials: F
authors.lastname: Paul
authors.affiliation: Department of Neurology, University of Heidelberg, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Jarius
authors.affiliation: Department of Neurology, Heinrich-Heine University, Germany.
authors.forename: O
authors.initials: O
authors.lastname: Aktas
authors.affiliation: The Walton Centre for Neurology and Neurosurgery, UK.
authors.forename: L
authors.initials: L
authors.lastname: Elsone
authors.affiliation: The Walton Centre for Neurology and Neurosurgery, UK.
authors.forename: K
authors.initials: K
authors.lastname: Mutch
authors.affiliation: Department of Neurology, John Hopkins University, USA.
authors.forename: M
authors.initials: M
authors.lastname: Levy
authors.affiliation: Department of Neurology, Tohoku University, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Takai
authors.affiliation: Department of Neurology, University Hospital of Strasbourg, France.
authors.forename: N
authors.initials: N
authors.lastname: Collongues
authors.affiliation: Division of Neurology, The Hospital for Sick Children, Toronto, Canada.
authors.forename: B
authors.initials: B
authors.lastname: Banwell
authors.affiliation: Department of Neurology, Tohoku University, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Fujihara
authors.affiliation: Department of Neurology, University Hospital of Strasbourg, France.
authors.forename: J
authors.initials: J
authors.lastname: de Seze
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D015415:Biomarkers / Q000097:blood; D001933:Brain Stem / Q000000981:diagnostic imaging / Q000276:immunology / Q000503:physiopathology*; D005060:Europe; D005260:Female; D006606:Hiccup / Q000175:diagnosis / Q000208:ethnology / Q000276:immunology / Q000503:physiopathology*; D006801:Humans; D007564:Japan; D008279:Magnetic Resonance Imaging; D008297:Male; D009471:Neuromyelitis Optica / Q000175:diagnosis / Q000208:ethnology / Q000276:immunology / Q000503:physiopathology*; D009656:North America; D015995:Prevalence; D012307:Risk Factors; D012698:Serologic Tests; D014839:Vomiting / Q000175:diagnosis / Q000208:ethnology / Q000276:immunology / Q000503:physiopathology*
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D015415:Biomarkers
keywords: Neuromyelitis optica; anti-aquaporin 4 antibodies; brainstem; demyelinating diseases; epidemiology; ethnicity